Songdej et al reported a score for identifying a pediatric patient with hemoglobin H who is likely to have severe disease requiring regular transfusions. The authors are from Ramathibodi Hospital, Mahidol University, and Chiangmai University Hospital in Thailand.
Patient selection: non-deletional hemoglobin H
Parameters:
(1) age at diagnosis in years
(2) spleen size in cm (probably extent below costal margin)
(3) hemoglobin at steady state
Parameter
Finding
Points
age at diagnosis
< 2 years of age
1
>= 2 years of age
0
spleen size
< 3 cm
0
>= 3 cm
1
hemoglobin
>= 8 g/dL
0
7 to 7.9 g/dL
3
< 7 g/dL
4
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 6
• A score >= 4 is associated with severe disease associated with regular transfusions.
Performance:
• A cutoff >=4 had a sensitivity of 0.89 and specificity of 0.81.
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